1Department of Internal Medicine, Yale New Haven Health, Bridgeport, Connecticut.
2Department of Neurosurgery, Feigenbaum Neurosurgery, Dallas, Texas.
J Neurosurg Spine. 2023 Dec 15;40(3):375-388. doi: 10.3171/2023.9.SPINE23559. Print 2024 Mar 1.
Tarlov perineurial spinal cysts (TCs) are an underrecognized cause of spinal neuropathic symptoms. TCs form within the sensory nerve root sleeves, where CSF extends distally and can accumulate pathologically. Typically, they develop at the sacral dermatomes where the nerve roots are under the highest hydrostatic pressure and lack enclosing vertebral foramina. In total, 90% of patients are women, and genetic disorders that weaken connective tissues, e.g., Ehlers-Danlos syndrome, convey considerable risk. Most small TCs are asymptomatic and do not require treatment, but even incidental visualizations should be documented in case symptoms develop later. Symptomatic TCs most commonly cause sacropelvic dermatomal neuropathic pain, as well as bladder, bowel, and sexual dysfunction. Large cysts routinely cause muscle atrophy and weakness by compressing the ventral motor roots, and multiple cysts or multiroot compression by one large cyst can cause even greater cauda equina syndromes. Rarely, giant cysts erode the sacrum or extend as intrapelvic masses. Disabling TCs require consideration for surgical intervention. The authors' systematic review of treatment analyzed 31 case series of interventional percutaneous procedures and open surgical procedures. The surgical series were smaller and reported somewhat better outcomes with longer term follow-up but slightly higher risks. When data were lacking, authorial expertise and case reports informed details of the specific interventional and surgical techniques, as well as medical, physical, and psychological management. Cyst-wrapping surgery appeared to offer the best long-term outcomes by permanently reducing cyst size and reconstructing the nerve root sleeves. This curtails ongoing injury to the axons and neuronal death, and may also promote axonal regeneration to improve somatic and autonomic sacral nerve function.
Tarlov 周围神经鞘瘤(TCs)是一种被低估的引起脊髓神经性症状的原因。TCs 形成于感觉神经根袖内,CSF 向远端延伸并在该处病理性积聚。它们通常发生在骶神经节段,神经根在此处承受最大的静水压力且缺乏包围的椎间孔。总体而言,90%的患者为女性,存在使结缔组织变弱的遗传疾病,例如埃勒斯-当洛斯综合征,会带来相当大的风险。大多数小的 TCs 无症状且无需治疗,但即使是偶然发现的,也应记录在案,以防日后出现症状。症状性 TCs 最常见的引起骶尾部皮节神经性疼痛,以及膀胱、肠道和性功能障碍。大的囊肿通常通过压迫腹侧运动神经根导致肌肉萎缩和无力,多个囊肿或一个大囊肿压迫多根神经根会导致更严重的马尾综合征。罕见情况下,巨大的囊肿侵蚀骶骨或扩展为盆腔内肿块。致残性 TCs 需要考虑手术干预。作者对治疗的系统回顾分析了 31 个介入性经皮手术和开放性手术系列。手术系列规模较小,且在长期随访时报告了稍好的结果,但风险略高。当数据缺乏时,作者的专业知识和病例报告提供了特定介入和手术技术的详细信息,以及医疗、物理和心理管理的细节。囊肿包裹手术似乎通过永久性减小囊肿大小并重建神经根袖提供了最佳的长期效果。这可以限制对轴突的持续损伤和神经元死亡,并可能促进轴突再生,以改善躯体和自主骶神经功能。